RESUMO
Aims:To describe a Axenfeld-Rieger Syndrome.Presentation of Case: MCL, 7 years old, female, brown, was taken to the ophthalmology outpatient clinic of the Hospital Universit醨io Ant鬾io Pedro, Brazil by her parents, complaining of low visual acuity and malformation of the pupil perceived since birth.Discussion: Axenfeld-Rieger Syndrome is a rare and hereditary disease. Clinically, Axenfeld's anomaly is characterized by the presence of posterior embryotoxon, and there may be adherence of iridian tissue in its periphery. In addition to Rieger's anomaly, posterior embryotoxon is added to iris hypoplasia and iris thickness defects, uveal ectropion and pupillary alterations, such as corectopia. Rieger's syndrome is associated with extraocular changes, of which hypodontia, myicrodontia, maxillary hypoplasia, telecanthus, hypertelorism and hypospadias stand out.Conclusions: Therefore, the importance of early diagnosis, follow-up and adequate treatment becomes evident in order to preserve the visual function of patients and thus avoid an unfavorable evolution.